Amyotrophic lateral sclerosis (ALS) is an incurable fatal motoneuron disease with a lifetime risk of 1:800. It is characterized by progressive weakness, muscle wasting and death ensuing 3-5 years after diagnosis. Currently, the only available treatment option is riluzole that prolongs life by 2-3 months, with questionable functional improvement. Accordingly, new compounds and methods for treating ALS are needed. The present invention addresses these needs.